Increased levels of CSF (4.0 g/L) were observed and anti-CNTN1 IgG4 antibodies were detected. However, 12 months later patient presented with nephrotic syndrome. Renal biopsy was suggestive of stage 2 MN; additionally the biopsy revealed excessive CNTN1 staining within glomerulus in comparison to controls. Here, CNTN1 is linked to nephrotic syndrome.